The Gift of Hearing

Like most new moms, Maryann Florio eagerly awaits each new milestone in her 2-year-old daughter’s young life. But for Florio, every hard-won achievement is all the more precious: a little more than a year ago, she learned that Annabella had profound hearing loss and was not responding to any sounds, let alone trying to repeat them. Over a period of weeks, however, Florio saw her daughter making huge strides in her language development.

“I almost cried when I said, ‘Annabella,’ and she turned around to look at me. It’s amazing. One day, your child isn’t hearing any sounds, and the next, there are so many possibilities,” Florio says.

The dramatic transformation is the result of surgical devices known as cochlear implants, which simulate hearing in individuals who have little to no hearing, and the work of the comprehensive Pediatric Cochlear Implant Team at Rutgers Health's Robert Wood Johnson Medical School and The Bristol-Myers Squibb Children’s Hospital at Robert Wood Johnson University Hospital.

Led by pediatric otolaryngologists Michael Chee and Kelvin M. Kwong, both assistant professors of surgery at Robert Wood Johnson Medical School, the highly specialized, multidisciplinary Pediatric Cochlear Implant Team also includes audiologists, speech/language pathologists, neuropsychologists, and a nurse coordinator and is one of the most comprehensive programs of its kind in the tristate region. Established in early 2014, it offers parents and children complete evaluation, treatment, and rehabilitation services related to cochlear implants.

At 4 months, Annabella was diagnosed with sensorineural hearing loss. This condition is the result of damage to the inner ear that prevents sound from traveling to the brain. A cochlear implant bypasses the inner ear, transmitting sound from an external speech processor and transmitter to an internal receiver that generates electrical pulses directly to the auditory nerve itself.

Annabella was 16 months old when the first cochlear device was implanted in her left ear in October 2014, and it was activated a month later; the remaining device was implanted in her right ear at the end of April 2015 and activated two months later.

“With the first implant, we noticed that, yes, she could hear something. But as soon as she got that second implant, it was like a switch was flipped and everything started to go really quickly,” Florio says. “She’s trying to say her ABCs, she knows about eight different colors and the noises of different animals—says ‘ooo’ for ‘moo’—and has really started catching up a lot quicker than what was expected. It’s wonderful. She’s babbling, making noises, and starting to sing. She knows the difference between when people are speaking and when they are singing. Every time she hears music, she stands up and dances.”

Annabella is involved in classes and therapy four days a week, including sessions with a speech therapist, as well as twice-weekly classes where she interacts with other children who have hearing loss. She sees a member of the cochlear implant team every few weeks for “mapping,” or adjustments to the sound programming of the device. Once mapping on both sides is complete, Annabella will return annually for any necessary tweaks to the programming.

“It can take approximately a year to stabilize the sound mapping so the implant can be customized to each child’s individual hearing loss,” explains Dr. Kwong. “While the surgery itself takes only a few hours, and most children are home the same day, it’s not just a simple procedure and then you’re done,” he adds. “The process is very labor intensive after the surgery, and a committed team is needed to help lay the groundwork for success.”

It takes dedication from the family and everyone else involved, agrees Dr. Chee. “It’s very intensive, but fortunately there is a lot of support from the team. They are not in it alone,” he says.

Clinical developmental psychologists with the Institute for the Study of Child Development in the medical school’s Department of Pediatrics work with the program to help families understand the developmental and cognitive potential of their young child, consider potential obstacles, and help them cope with the stresses and anxieties associated with the procedure. After surgery, they also are available to assist as children learn to understand new sounds and adjust to life with their implant.

“With the advent of universal hearing screening, there is no reason for a child to have profound hearing loss and not at least be offered a chance for hearing,” says Dr. Chee.

This Rutgers Health article is an edited version of a story that first appeared in the Fall 2015 edition of Robert Wood Johnson Medical School's Robert Wood Johnson Medicine. [Read the full story]